An infant with failure to thrive, hypertension, metabolic alkalosis and hyperkalemia presents to a clinician. Most probable cause is

Correct Answer: Gordon syndrome
Description: d. Gordon syndrome(Ref: Nelson 20/e p 2535)Out of the given causes, hyperkalemia is seen only in Gordon syndromeGordon syndrome or familial hyperkalemic hypertension, is due to gain-of-function mutations in WNK1 & ioss-of-funotion mutations in WNK4 in distal convoluted tubule, leading to excessive NCCT-mediated salt reabsorption with the clinical picture of pseudohypoaldosteronism type 2Liddle syndrome is due to gain-of-function mutations of the gene that encodes the epithelial sodium channel in the collecting duct, leading to hypertension, hypokalemia & suppressed aldosterone
Category: Pediatrics
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